MCID: LMB053
MIFTS: 27

Limbic Encephalitis with Lgi1 Antibodies

Categories: Rare diseases, Neuronal diseases, Immune diseases

Aliases & Classifications for Limbic Encephalitis with Lgi1 Antibodies

MalaCards integrated aliases for Limbic Encephalitis with Lgi1 Antibodies:

Name: Limbic Encephalitis with Lgi1 Antibodies 53 59
Limbic Encephalitis with Leucine-Rich Glioma-Inactivated 1 Antibodies 53 59

Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

Orphanet 59 ORPHA163908
ICD10 via Orphanet 34 G13.1

Summaries for Limbic Encephalitis with Lgi1 Antibodies

NIH Rare Diseases : 53 Limbic encephalitis with LGI1 antibodies is a relatively new disease characterized by progressive memory loss, confusion, sleep disturbances, and problems with behaviors and spatial orientation. Seizures are common and frequently precede other symptoms. About half of people with this diagnosis exhibit a specific type of seizure called faciobrachial dystonic seizures, which resemble brief jerks of the face arm.Hyponatremia (low levels of sodium in the blood) may also be present. The disease occurs when antibodies mistakenly attack LGI1 (leucine-rich glioma inactivated 1), a protein found in the brain that binds together two epilepsy-related proteins called ADAM22 and ADAM23. Some people with limbic encephalitis with LGI1 antibodies (5 to 10%) also have a cancer diagnosis (most commonly thymoma and lung cancer), but most do not.  The disease is diagnosed through testing of blood or spinal fluid for LGI1 antibodies. If treatment is started when seizures are first noticed, limbic encephalitis may be prevented. Treatment is intravenous or oral corticosteroids, intravenous immunoglobulin (IVIG), plasmapheresis (a process where antibodies are removed from the blood), or a combination of these. Long-term outcome is mostly favorable, although some people are left with some degree of residual impairment. Relapse occurs in up to one third of patients.

MalaCards based summary : Limbic Encephalitis with Lgi1 Antibodies, also known as limbic encephalitis with leucine-rich glioma-inactivated 1 antibodies, is related to encephalitis and limbic encephalitis. An important gene associated with Limbic Encephalitis with Lgi1 Antibodies is HLA-DRB1 (Major Histocompatibility Complex, Class II, DR Beta 1), and among its related pathways/superpathways are Actin Nucleation by ARP-WASP Complex and Influenza A. Affiliated tissues include brain, testes and lung.

Related Diseases for Limbic Encephalitis with Lgi1 Antibodies

Diseases related to Limbic Encephalitis with Lgi1 Antibodies via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 37)
# Related Disease Score Top Affiliating Genes
1 encephalitis 10.3
2 limbic encephalitis 10.3
3 metal allergy 9.8 HLA-DQB1 HLA-DRB1
4 focal epithelial hyperplasia 9.8 HLA-DQB1 HLA-DRB1
5 hypersomnia 9.8 HLA-DQB1 HLA-DRB1
6 lichen planopilaris 9.8 HLA-DQB1 HLA-DRB1
7 type ii mixed cryoglobulinemia 9.8 HLA-DQB1 HLA-DRB1
8 beryllium disease 9.8 HLA-DQB1 HLA-DRB1
9 recurrent respiratory papillomatosis 9.8 HLA-DQB1 HLA-DRB1
10 autoimmune pancreatitis 9.8 HLA-DQB1 HLA-DRB1
11 chronic beryllium disease 9.8 HLA-DQB1 HLA-DRB1
12 pityriasis rosea 9.8 HLA-DQB1 HLA-DRB1
13 lichen sclerosus 9.8 HLA-DQB1 HLA-DRB1
14 whipple disease 9.8 HLA-DQB1 HLA-DRB1
15 acute disseminated encephalomyelitis 9.8 HLA-DQB1 HLA-DRB1
16 pediatric multiple sclerosis 9.8 HLA-DQB1 HLA-DRB1
17 osteonecrosis of the jaw 9.8 HLA-DQB1 HLA-DRB1
18 idiopathic bronchiectasis 9.8 HLA-DQB1 HLA-DRB1
19 peanut allergy 9.7 HLA-DQB1 HLA-DRB1
20 oligoarticular juvenile idiopathic arthritis 9.7 HLA-DQB1 HLA-DRB1
21 panuveitis 9.7 HLA-DQB1 HLA-DRB1
22 geographic tongue 9.7 HLA-DQB1 HLA-DRB1
23 severe hemophilia a 9.7 HLA-DQB1 HLA-DRB1
24 ocular cicatricial pemphigoid 9.7 HLA-DQB1 HLA-DRB1
25 paraneoplastic pemphigus 9.7 HLA-DQB1 HLA-DRB1
26 vogt-koyanagi-harada disease 9.7 HLA-DQB1 HLA-DRB1
27 rubella 9.6 HLA-DQB1 HLA-DRB1
28 graft-versus-host disease 9.6 HLA-DQB1 HLA-DRB1
29 autoimmune hepatitis 9.6 HLA-DQB1 HLA-DRB1
30 bullous pemphigoid 9.6 HLA-DQB1 HLA-DRB1
31 leishmaniasis 9.6 HLA-DQB1 HLA-DRB1
32 narcolepsy 9.5 HLA-DQB1 HLA-DRB1
33 diabetes mellitus, insulin-dependent 9.5 HLA-DQB1 HLA-DRB1
34 arthritis 9.4 HLA-DQB1 HLA-DRB1
35 autoimmune disease 9.3 HLA-DQB1 HLA-DRB1
36 multiple sclerosis 9.2 HLA-DQB1 HLA-DRB1
37 rheumatoid arthritis 9.0 HLA-DQB1 HLA-DRB1

Graphical network of the top 20 diseases related to Limbic Encephalitis with Lgi1 Antibodies:



Diseases related to Limbic Encephalitis with Lgi1 Antibodies

Symptoms & Phenotypes for Limbic Encephalitis with Lgi1 Antibodies

Drugs & Therapeutics for Limbic Encephalitis with Lgi1 Antibodies

Search Clinical Trials , NIH Clinical Center for Limbic Encephalitis with Lgi1 Antibodies

Genetic Tests for Limbic Encephalitis with Lgi1 Antibodies

Anatomical Context for Limbic Encephalitis with Lgi1 Antibodies

MalaCards organs/tissues related to Limbic Encephalitis with Lgi1 Antibodies:

41
Brain, Testes, Lung

Publications for Limbic Encephalitis with Lgi1 Antibodies

Articles related to Limbic Encephalitis with Lgi1 Antibodies:

# Title Authors Year
1
Limbic encephalitis with LGI1 antibodies in a 14-year-old boy. ( 28919330 )
2017
2
Selective Limbic Blood-Brain Barrier Breakdown in a Feline Model of Limbic Encephalitis with LGI1 Antibodies. ( 29093718 )
2017
3
Clinical features of limbic encephalitis with LGI1 antibody. ( 28670128 )
2017

Variations for Limbic Encephalitis with Lgi1 Antibodies

Expression for Limbic Encephalitis with Lgi1 Antibodies

Search GEO for disease gene expression data for Limbic Encephalitis with Lgi1 Antibodies.

Pathways for Limbic Encephalitis with Lgi1 Antibodies

Pathways related to Limbic Encephalitis with Lgi1 Antibodies according to GeneCards Suite gene sharing:

(show all 23)
# Super pathways Score Top Affiliating Genes
1
Show member pathways
12.44 HLA-DQB1 HLA-DRB1
2
Show member pathways
12.29 HLA-DQB1 HLA-DRB1
3
Show member pathways
12.29 HLA-DQB1 HLA-DRB1
4
Show member pathways
12.14 HLA-DQB1 HLA-DRB1
5
Show member pathways
12.08 HLA-DQB1 HLA-DRB1
6
Show member pathways
12 HLA-DQB1 HLA-DRB1
7 11.97 HLA-DQB1 HLA-DRB1
8
Show member pathways
11.94 HLA-DQB1 HLA-DRB1
9
Show member pathways
11.9 HLA-DQB1 HLA-DRB1
10 11.84 HLA-DQB1 HLA-DRB1
11
Show member pathways
11.8 HLA-DQB1 HLA-DRB1
12 11.76 HLA-DQB1 HLA-DRB1
13
Show member pathways
11.73 HLA-DQB1 HLA-DRB1
14 11.66 HLA-DQB1 HLA-DRB1
15
Show member pathways
11.64 HLA-DQB1 HLA-DRB1
16 11.59 HLA-DQB1 HLA-DRB1
17 11.43 HLA-DQB1 HLA-DRB1
18
Show member pathways
11.41 HLA-DQB1 HLA-DRB1
19 11.33 HLA-DQB1 HLA-DRB1
20 11.24 HLA-DQB1 HLA-DRB1
21 11.11 HLA-DQB1 HLA-DRB1
22 10.81 HLA-DQB1 HLA-DRB1
23 10.37 HLA-DQB1 HLA-DRB1

GO Terms for Limbic Encephalitis with Lgi1 Antibodies

Cellular components related to Limbic Encephalitis with Lgi1 Antibodies according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 lysosomal membrane GO:0005765 9.46 HLA-DQB1 HLA-DRB1
2 endosome membrane GO:0010008 9.43 HLA-DQB1 HLA-DRB1
3 trans-Golgi network membrane GO:0032588 9.4 HLA-DQB1 HLA-DRB1
4 endocytic vesicle membrane GO:0030666 9.37 HLA-DQB1 HLA-DRB1
5 ER to Golgi transport vesicle membrane GO:0012507 9.32 HLA-DQB1 HLA-DRB1
6 transport vesicle membrane GO:0030658 9.26 HLA-DQB1 HLA-DRB1
7 clathrin-coated endocytic vesicle membrane GO:0030669 9.16 HLA-DQB1 HLA-DRB1
8 integral component of lumenal side of endoplasmic reticulum membrane GO:0071556 8.96 HLA-DQB1 HLA-DRB1
9 MHC class II protein complex GO:0042613 8.62 HLA-DQB1 HLA-DRB1

Biological processes related to Limbic Encephalitis with Lgi1 Antibodies according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 immune response GO:0006955 9.46 HLA-DQB1 HLA-DRB1
2 T cell receptor signaling pathway GO:0050852 9.43 HLA-DQB1 HLA-DRB1
3 antigen processing and presentation of exogenous peptide antigen via MHC class II GO:0019886 9.4 HLA-DQB1 HLA-DRB1
4 T cell costimulation GO:0031295 9.37 HLA-DQB1 HLA-DRB1
5 interferon-gamma-mediated signaling pathway GO:0060333 9.32 HLA-DQB1 HLA-DRB1
6 antigen processing and presentation GO:0019882 9.26 HLA-DQB1 HLA-DRB1
7 antigen processing and presentation of peptide or polysaccharide antigen via MHC class II GO:0002504 9.16 HLA-DQB1 HLA-DRB1
8 humoral immune response mediated by circulating immunoglobulin GO:0002455 8.96 HLA-DQB1 HLA-DRB1
9 immunoglobulin production involved in immunoglobulin mediated immune response GO:0002381 8.62 HLA-DQB1 HLA-DRB1

Molecular functions related to Limbic Encephalitis with Lgi1 Antibodies according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 peptide antigen binding GO:0042605 8.96 HLA-DQB1 HLA-DRB1
2 MHC class II receptor activity GO:0032395 8.62 HLA-DQB1 HLA-DRB1

Sources for Limbic Encephalitis with Lgi1 Antibodies

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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